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Abnormal earlobe morphology

MedGen UID:
867434
•Concept ID:
C4021808
•
Anatomical Abnormality
Synonym: Abnormality of earlobe
 
HPO: HP:0000363

Definition

An abnormality of the lobule of pinna. [from HPO]

Conditions with this feature

Syndactyly-polydactyly-ear lobe syndrome
MedGen UID:
348333
•Concept ID:
C1861347
•
Disease or Syndrome
A rare, genetic, congenital limb malformation syndrome characterized by complete cutaneous syndactyly between toes 1-2, ulnar polydactyly (ranging from nubbins to an almost complete additional finger) and earlobe malformations. Additionally, abnormalities along the medial border of the foot are observed on X-ray imaging. There have been no further descriptions in the literature since 1976.
See: Condition Record
Syndactyly-polydactyly-ear lobe syndrome

Professional guidelines

PubMed

Novel phenotypes and genotypes in Antley-Bixler syndrome caused by cytochrome P450 oxidoreductase deficiency: based on the first cohort of Chinese children.
Fan L, Ren X, Song Y, Su C, Fu J, Gong C
Orphanet J Rare Dis 2019 Dec 30;14(1):299. doi: 10.1186/s13023-019-1283-2. PMID: 31888681Free PMC Article
Clinical features and management issues in Mowat-Wilson syndrome.
Adam MP, Schelley S, Gallagher R, Brady AN, Barr K, Blumberg B, Shieh JT, Graham J, Slavotinek A, Martin M, Keppler-Noreuil K, Storm AL, Hudgins L
Am J Med Genet A 2006 Dec 15;140(24):2730-41. doi: 10.1002/ajmg.a.31530. PMID: 17103451

Recent clinical studies

Etiology

Surgical correction of pixie-ear defect without an exposed incision on the lobe.
Laurence VG, Wirth GA, Michaud JP, Wood DL
Ann Plast Surg 2012 Apr;68(4):360-1. doi: 10.1097/SAP.0b013e31824eefab. PMID: 22421478
Earlobe reconstruction using a Limberg flap in six ears.
Singh A, Singh G
Br J Plast Surg 2003 Jan;56(1):33-6. doi: 10.1016/s0007-1226(03)00002-x. PMID: 12706147
Diagonal earlobe creases and prognosis in patients with suspected coronary artery disease.
Elliott WJ, Powell LH
Am J Med 1996 Feb;100(2):205-11. doi: 10.1016/s0002-9343(97)89460-0. PMID: 8629656
Cleft earlobe repair.
Fearon J, Cuadros CL
Ann Plast Surg 1990 Mar;24(3):252-7. doi: 10.1097/00000637-199003000-00010. PMID: 2316987
Letter: Diagonal ear-lobe crease and coronary artery sclerosis.
Lichtstein E, Chapman I, Gupta PK, Chadda KD, Smith H Jr, Schwartz I, Naik D
Ann Intern Med 1976 Sep;85(3):337-8. doi: 10.7326/0003-4819-85-3-337. PMID: 962224

Diagnosis

Possible new defining presentation of mosaic tetrasomy 9p: multiple and recurrent pilomatrixoma.
Wang JW, Behnam R, Porto DA
Dermatol Online J 2024 Jul 15;30(3) doi: 10.5070/D330363866. PMID: 39090039
Cutaneous Mucinosis Associated with Beckwith-Wiedemann Syndrome.
Lacey R, Shalabi D, Nikbakht N
Skinmed 2020;18(1):42-44. Epub 2020 Jan 1 PMID: 32167456
Sheldon-Hall syndrome.
Toydemir RM, Bamshad MJ
Orphanet J Rare Dis 2009 Mar 23;4:11. doi: 10.1186/1750-1172-4-11. PMID: 19309503Free PMC Article
Topics in pediatric plastic surgery.
Denk MJ
Pediatr Clin North Am 1998 Dec;45(6):1479-506, ix. doi: 10.1016/s0031-3955(05)70100-0. PMID: 9889763
Diagonal earlobe creases and prognosis in patients with suspected coronary artery disease.
Elliott WJ, Powell LH
Am J Med 1996 Feb;100(2):205-11. doi: 10.1016/s0002-9343(97)89460-0. PMID: 8629656

Therapy

Incomplete Cleft Earlobe Reconstruction Using a Z-plasty or Hammock Flap.
Vargas-Laguna E, Imbernón-Moya A, Lobato Berezo A
Actas Dermosifiliogr 2016 Nov;107(9):775-776. Epub 2016 Jul 4 doi: 10.1016/j.ad.2016.05.013. PMID: 27389184
Prevention of earlobe deformity in face-lifting.
Coscia J, Huang A, Stevenson TR
Ann Plast Surg 2013 Apr;70(4):389-91. doi: 10.1097/SAP.0b013e31828a0cbd. PMID: 23486143
Earlobe rejuvenation.
Hotta T
Plast Surg Nurs 2011 Jan-Mar;31(1):39-40. doi: 10.1097/PSN.0b013e31820f53fc. PMID: 21368648
Protracted superficial Wegener's granulomatosis.
Sinovich V, Snow J
Australas J Dermatol 2003 Aug;44(3):207-14. doi: 10.1046/j.1440-0960.2003.00689.x. PMID: 12869048
Plastic and reconstructive surgery of the earlobe.
Clevens RA, Baker SR
Facial Plast Surg 1995 Oct;11(4):301-9. doi: 10.1055/s-2008-1064546. PMID: 9046618

Prognosis

Fryns syndrome: case report and review of the literature.
Yucesoy G, Cakiroglu Y, Caliskan E
J Clin Ultrasound 2008 Jun;36(5):315-7. doi: 10.1002/jcu.20409. PMID: 17960800
Earlobe reconstruction using a Limberg flap in six ears.
Singh A, Singh G
Br J Plast Surg 2003 Jan;56(1):33-6. doi: 10.1016/s0007-1226(03)00002-x. PMID: 12706147
A newly defined conchal floor arterial flap for auricular reconstruction.
Park C
Plast Reconstr Surg 2002 Jul;110(1):47-53. doi: 10.1097/00006534-200207000-00010. PMID: 12087230
Earlobe reconstruction with a reverse-flow chondrocutaneous flap.
Van Geertruyden JP
Br J Plast Surg 2002 Apr;55(3):253-5. doi: 10.1054/bjps.2002.3817. PMID: 12041982
Diagonal earlobe creases and prognosis in patients with suspected coronary artery disease.
Elliott WJ, Powell LH
Am J Med 1996 Feb;100(2):205-11. doi: 10.1016/s0002-9343(97)89460-0. PMID: 8629656

Clinical prediction guides

Phenotypic variations of the human ear in the Basrah population.
Hassan KK, Ali SM, Saleem RI
Otolaryngol Pol 2025 Feb 26;79(2):1-7. doi: 10.5604/01.3001.0055.0000. PMID: 40008474
Prograde transposition for anteriorly low-set earlobe in lobule-type microtia reconstruction.
Deng Y, Yang Y, Wang T, Wang B, Wang P, Zhi J, Xu D, Jiang H
J Plast Reconstr Aesthet Surg 2024 May;92:145-150. Epub 2024 Mar 5 doi: 10.1016/j.bjps.2024.02.073. PMID: 38518626
A Different Approach for Protruding Earlobe Correction-Modified Fish-tail Technique.
Sert B, Oral MA, Turan O, Uyar I
Aesthetic Plast Surg 2024 Jun;48(11):2011-2017. Epub 2024 Mar 4 doi: 10.1007/s00266-024-03912-7. PMID: 38438764Free PMC Article
Diagonal earlobe creases and prognosis in patients with suspected coronary artery disease.
Elliott WJ, Powell LH
Am J Med 1996 Feb;100(2):205-11. doi: 10.1016/s0002-9343(97)89460-0. PMID: 8629656
Kabuki make-up syndrome: a syndrome of mental retardation, unusual facies, large and protruding ears, and postnatal growth deficiency.
Niikawa N, Matsuura N, Fukushima Y, Ohsawa T, Kajii T
J Pediatr 1981 Oct;99(4):565-9. doi: 10.1016/s0022-3476(81)80255-7. PMID: 7277096

Recent systematic reviews

Earlobe Correction of the Pierced Ear: A Systematic Review of the Literature and Principles for Surgical Reconstruction.
Grohmann M, Weiland T, Tuca AC, Wimbauer JM
Facial Plast Surg Aesthet Med 2023 Mar-Apr;25(2):83-89. Epub 2022 Feb 9 doi: 10.1089/fpsam.2021.0269. PMID: 35138923

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